BIOMAT Database Logo BIOMAT Database Logo

Iron therapy for hepatic dysfunction in erythropoietic protoporphyria. 1986

V R Gordeuk, and G M Brittenham, and C W Hawkins, and H Mukhtar, and D R Bickers

Erythropoietic protoporphyria, caused by an inherited deficiency of ferrochelatase, results in increased free protoporphyrin concentrations in erythrocytes and plasma. Cutaneous photosensitivity is the primary clinical manifestation, but in some patients, protoporphyrin accumulation in the liver may lead to fatal hepatic failure. No treatment exists for this lethal complication. We report the case of a patient with erythropoietic protoporphyria and abnormal results of liver function tests in whom oral iron therapy led to a substantial decrease in free erythrocyte and stool protoporphyrin levels with return of liver function to normal. Iron therapy should be considered for preventing potentially fatal hepatic failure in patients with erythropoietic protoporphyria.

UI MeSH Term Description Entries
D007501 Iron A metallic element with atomic symbol Fe, atomic number 26, and atomic weight 55.85. It is an essential constituent of HEMOGLOBINS; CYTOCHROMES; and IRON-BINDING PROTEINS. It plays a role in cellular redox reactions and in the transport of OXYGEN. Iron-56,Iron 56
D008107 Liver Diseases Pathological processes of the LIVER. Liver Dysfunction,Disease, Liver,Diseases, Liver,Dysfunction, Liver,Dysfunctions, Liver,Liver Disease,Liver Dysfunctions
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009942 Organometallic Compounds A class of compounds of the type R-M, where a C atom is joined directly to any other element except H, C, N, O, F, Cl, Br, I, or At. (Grant & Hackh's Chemical Dictionary, 5th ed) Metallo-Organic Compound,Metallo-Organic Compounds,Metalloorganic Compound,Organometallic Compound,Metalloorganic Compounds,Compound, Metallo-Organic,Compound, Metalloorganic,Compound, Organometallic,Compounds, Metallo-Organic,Compounds, Metalloorganic,Compounds, Organometallic,Metallo Organic Compound,Metallo Organic Compounds
D010787 Photosensitivity Disorders Abnormal responses to sunlight or artificial light due to extreme reactivity of light-absorbing molecules in tissues. It refers almost exclusively to skin photosensitivity, including sunburn, reactions due to repeated prolonged exposure in the absence of photosensitizing factors, and reactions requiring photosensitizing factors such as photosensitizing agents and certain diseases. With restricted reference to skin tissue, it does not include photosensitivity of the eye to light, as in photophobia or photosensitive epilepsy. Actinic Reticuloid Syndrome,Dermatitis, Actinic,Photodermatitis,Chronic Actinic Dermatitis,Photosensitization,Actinic Dermatitides,Actinic Dermatitides, Chronic,Actinic Dermatitis,Actinic Dermatitis, Chronic,Actinic Reticuloid Syndromes,Chronic Actinic Dermatitides,Dermatitides, Actinic,Dermatitides, Chronic Actinic,Dermatitis, Chronic Actinic,Disorder, Photosensitivity,Disorders, Photosensitivity,Photodermatitides,Photosensitivity Disorder,Reticuloid Syndrome, Actinic,Reticuloid Syndromes, Actinic,Syndrome, Actinic Reticuloid,Syndromes, Actinic Reticuloid
D011164 Porphyrias A diverse group of metabolic diseases characterized by errors in the biosynthetic pathway of HEME in the LIVER, the BONE MARROW, or both. They are classified by the deficiency of specific enzymes, the tissue site of enzyme defect, or the clinical features that include neurological (acute) or cutaneous (skin lesions). Porphyrias can be hereditary or acquired as a result of toxicity to the hepatic or erythropoietic marrow tissues. Porphyria,Porphyrin Disorder,Disorder, Porphyrin,Disorders, Porphyrin,Porphyrin Disorders
D011166 Porphyrins A group of compounds containing the porphin structure, four pyrrole rings connected by methine bridges in a cyclic configuration to which a variety of side chains are attached. The nature of the side chain is indicated by a prefix, as uroporphyrin, hematoporphyrin, etc. The porphyrins, in combination with iron, form the heme component in biologically significant compounds such as hemoglobin and myoglobin. Porphyrin
D011524 Protoporphyrins Porphyrins with four methyl, two vinyl, and two propionic acid side chains attached to the pyrrole rings. Protoporphyrin IX occurs in hemoglobin, myoglobin, and most of the cytochromes.
D004912 Erythrocytes Red blood cells. Mature erythrocytes are non-nucleated, biconcave disks containing HEMOGLOBIN whose function is to transport OXYGEN. Blood Cells, Red,Blood Corpuscles, Red,Red Blood Cells,Red Blood Corpuscles,Blood Cell, Red,Blood Corpuscle, Red,Erythrocyte,Red Blood Cell,Red Blood Corpuscle
D005243 Feces Excrement from the INTESTINES, containing unabsorbed solids, waste products, secretions, and BACTERIA of the DIGESTIVE SYSTEM.

Related Publications

V R Gordeuk, and G M Brittenham, and C W Hawkins, and H Mukhtar, and D R Bickers
Erythropoietic protoporphyria and iron therapy.
March 1990, The British journal of dermatology,
V R Gordeuk, and G M Brittenham, and C W Hawkins, and H Mukhtar, and D R Bickers
Erythropoietic protoporphyria provoked by iron therapy.
June 1971, Proceedings of the Royal Society of Medicine,
V R Gordeuk, and G M Brittenham, and C W Hawkins, and H Mukhtar, and D R Bickers
Erythropoietic protoporphyria exacerbated by oral iron therapy.
July 1988, The British journal of dermatology,
V R Gordeuk, and G M Brittenham, and C W Hawkins, and H Mukhtar, and D R Bickers
Erythropoietic protoporphyria: hepatic cirrhosis.
March 1978, The British journal of dermatology,
V R Gordeuk, and G M Brittenham, and C W Hawkins, and H Mukhtar, and D R Bickers
Erythropoietic protoporphyria. Hepatic implications.
August 1976, The British journal of dermatology,
V R Gordeuk, and G M Brittenham, and C W Hawkins, and H Mukhtar, and D R Bickers
Hepatic disease in erythropoietic protoporphyria.
June 1975, The American journal of medicine,
V R Gordeuk, and G M Brittenham, and C W Hawkins, and H Mukhtar, and D R Bickers
Hepatic disease in erythropoietic protoporphyria.
January 1986, Dermatologica,
V R Gordeuk, and G M Brittenham, and C W Hawkins, and H Mukhtar, and D R Bickers
PUVA therapy for erythropoietic protoporphyria.
June 1988, Photo-dermatology,
V R Gordeuk, and G M Brittenham, and C W Hawkins, and H Mukhtar, and D R Bickers
Transfusion therapy for erythropoietic protoporphyria.
November 1983, The British journal of dermatology,
V R Gordeuk, and G M Brittenham, and C W Hawkins, and H Mukhtar, and D R Bickers
Hepatic complications of erythropoietic protoporphyria.
April 1998, Photodermatology, photoimmunology & photomedicine,
Copied contents to your clipboard!